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Image of the month: Clinically suspected cardiac sarcoidosis

01 Jan 2014

Dr. Fernando Macaya

Dr. Grau Andrés

Dr. González Rosa

Dr. Expósito Carmen

We present the case of a 31-year-old male who suffered an episode of sudden death that was resuscitated, with ventricular fibrillation recorded as the first rhythm. Basal electrocardiogram showed negative T waves in inferior and left precordial leads (see figure 1).

Coronary angiography was performed showing no lesions. Transthoracic echocardiogram showed normal left ventricular function with mild septal hypertrophy (13 mm) and no other abnormalities. Cardiac magnetic resonance (CMR) showed a patchy pattern of intramyocardial late gadolinium enhancement in basal regions of anterior and lateral wall, medium and distal septum, inferior wall, as well as the free wall of the right ventricle (see figure 2 yellow arrowheads), all together strongly suggestive of cardiac sarcoidosis.

Endomyocardial biopsy performed was not pathologic. Thorax CT scan did not show any abnormalities. With no evidence of extracardiac sarcoidosis, no other tests were performed and Implantable Cardioverter Defibrillator (ICD) was implanted, beta blockers initiated and patient discharged.

A year later was admitted because of several episodes of ventricular fibrillation, treated by the ICD. Echocardiogram and electrocardiogram showed the same findings. Serum angiotensin-converting enzyme was 105 U/L (normal below 52 U/L). A cardiac and whole body scintigraphy was scheduled. Gallium-67 uptake was slightly increased in a patchy manner by heart muscle, with a remarkable apical enhancement, close to the ICD electrode, likely to do with the injury secondary to the discharges of the dispositive. A six-millimeter preaortic adenopathy showed discrete gallium uptake. There was no uptake in other sites.

Given the poor diagnostic yield of endomyocardial biopsy and the unfeasibility of extracardiac histological confirmation (anatomical difficulties to biopsy the adenopathy), diagnosis of isolated cardiac sarcoidosis was made based on multimodality imaging, following stated criteria (see readings below), considering an abnormal electrocardiogram, regional wall morphological abnormality, late gadolinium enhancement with typical pattern in CMR and mild gallium-67 myocardial uptake.

Methotrexate was initiated in combination of low doses of prednisone, slowly tapered within a year. Amiodarone and beta blocker were sustained as basal treatment. One year later, the patient remained asymptomatic and no more electrical events were recorded by the ICD. Echocardiogram showed no changes, with preserved systolic left ventricular function.

Conclusion:

We propose here a non-invasive approach (based on published criteria) for cases were cardiac sarcoidosis is suspected and extracardiac histological confirmation cannot be performed, intending to provide a prompt medical treatment to the patient. We also suggest taking into account cardiac-PET or PET-CT, that could play an important role revealing focal inflammation in cardiac sarcoidosis and locating extracardiac sites for biopsy. In our case this examination was dismissed because was not available in that moment.

Editorial comment:

In this case, based upon clinical and non-invasive findings and the response to immunosuppressive therapy, a diagnosis of clinically suspected cardiac sarcoidosis was done. We acknowledge that the management could not be shared by other teams since histological proof of isolated cardiac sarcoidosis is considered mandatory before specific treatment is considered. However this case illustrates the complexity of daily practice.